Intracranial Ganglioglioma Research Articles

Radiological and clinical outcomes of stereotactic radiosurgery for gangliogliomas: an international multicenter study.

The optimal treatment for recurrent and residual gangliogliomas remains unclear. The aim of this study was to evaluate the safety and efficacy of stereotactic radiosurgery (SRS) in the management of patients with recurrent or residual intracranial ganglioglioma. This retrospective multicenter study involved patients managed with SRS for ganglioglioma. The study endpoints included local tumor control and tumor- or SRS-related neurological morbidity following treatment. Factors associated with tumor progression and neurological morbidity were also analyzed. The cohort included 20 patients (11 males [55%]) with a median age of 24.5 (IQR 14) years who had been managed with SRS for ganglioglioma. Five-year radiological progression-free survival was 85.6%. After SRS, 2 patients (10%) experienced transient neurological deterioration. At a median clinical follow-up of 88.5 (IQR 112.5) months, 1 patient (5%) experienced seizure worsening and 1 (5%) required further resection of the tumor because of radiological progression. No mortality was noted in this series. SRS appears to be a safe and effective treatment option for surgically inaccessible, recurrent, and residual gangliogliomas. In this series, the 5-year progression-free survival rate after SRS was 85.6%. Gross-total resection remains the primary treatment of choice for patients with newly diagnosed or recurrent ganglioglioma. SRS may be considered for patients unfit for surgery and those with surgically inaccessible newly diagnosed, residual, and recurrent lesions.

Intracranial ganglioglioma WHO I: results in a series of eight patients treated with stereotactic interstitial brachytherapy.

In this retrospective study we evaluated the efficacy of interstitial brachytherapy (IBT) using (125)Iodine seeds ((125)I) for intracranial ganglioglioma WHO I. Between October 1994 and March 2010, eight patients (m/f = 5/3, median age 30.4 years, age range 7-42.5 years) with intracranial ganglioglioma WHO I were treated with IBT using stereotactically implanted (125)I seeds. The median follow-up time was 41.5 months (range 16.7-140.1 months). Prior to interstitial brachytherapy one patient underwent microsurgical resection for three times; seven patients were treated with IBT primarily. In all patients we implanted the (125)I seeds stereotactically guided. The cumulative tumor surface dose ranged between 50 and 65 Gy (permanent implantation) and the median tumor volume was 5.6 ml (range 0.9-26 ml). After brachytherapy, follow-up MR imaging revealed complete remission in one patient, partial remission in three and stable disease in the remaining four patients. Five of eight patients presented with seizures were either seizure-free (1/5) or improved (4/5). Temporary treatment-related morbidity occurred in one patient only (headache, nausea/vomiting) and resolved completely under steroid medication after 4 weeks. No treatment-related mortality was observed. This study indicates that interstitial brachytherapy for the treatment of intracranial ganglioglioma WHO I is safe and provides a high rate of local tumor control. Due to the limited number of cases, it is not possible to conduct a rigorous statistical evaluation. Thus, larger numbers of patients are required.

Treatment and outcomes for gangliogliomas: a single-center review of 16 patients.

BackgroundGanglioglioma is a rare and slowly growing benign tumor. We investigated the outcomes of patients who underwent different combination treatments.MethodsBetween 1998 and 2012, 16 patients, including 11 men and 5 women, with a median age of 12.5 years (range, 2.5-65 years) were treated for intracranial gangliogliomas at our institution. The median follow-up period was 5.7 years (range, 48 days-15.6 years). Fifteen cases were included in the outcome assessment because one patient was lost to follow-up. Complete resection was achieved in 8 (53%) patients. Six (40%) patients underwent incomplete resection with or without adjuvant radiotherapy, and one patient with a brainstem tumor underwent only stereotactic biopsy.ResultsGangliogliomas predominantly affected young (87.5%), male patients and most frequently presented with seizures (64%). Of eight patients who underwent complete resection, seven did not show recurrence, whereas only three of six with incomplete resection showed no recurrence. Four patients with recurrence received salvage treatments (two repeat surgeries and two radiosurgeries). A tumor control rate of 93% (14/15) was achieved at the last follow-up. No recurrence or malignant changes were observed after a median follow-up of 12 and 4.5 years in four patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment.ConclusionComplete resection produced the best outcomes and incomplete resection followed by adjuvant or salvage treatments showed favorable outcomes. In patients who are not eligible for complete resection because of tumor location or potential neurologic deficits following surgery, GK radiosurgery should be considered for the treatment of residual or recurrent tumors.

Long-term outcomes for low-grade intracranial ganglioglioma: 30-year experience from the Mayo Clinic

Gangliogliomas comprise less than 1% of all brain tumors and occur most often in children. Therefore, there are a limited number of patients and data involving the use or role of adjuvant therapy after subtotal resections (STRs) of gangliogliomas. The objective of this study was to examine and review the Mayo Clinic experience of 88 patients with gangliogliomas, their follow-up, risk of recurrence, and the role of radiation therapy after STR or only biopsy. Eighty-eight patients with gangliogliomas diagnosed between 1970 and 2007 were reviewed. Data on clinical outcomes and therapy received were analyzed. The Kaplan-Meier method was used to estimate progression-free survival (PFS) and overall survival. The median age at diagnosis was 19 years. The median potential follow-up as of June 2008 was 142 months (range 9-416 months). Fifteen-year overall survival was 94%, median PFS was 5.6 years, with a 10-year PFS rate of 37%. Progression-free survival was dramatically affected by extent of initial resection (p < 0.0001). This single-institution retrospective series of patients with gangliogliomas is unique given its large cohort size with a long follow-up duration, and confirms the excellent long-term survival rate in this group. The study also shows the importance of resection extent on likelihood of recurrence. Patients with gangliogliomas who undergo STR or biopsy alone have poor PFS. Radiation therapy may delay time to progression in patients with unresectable disease.

Intracranial gangliogliomas. A review of a series of 20 patients

Introduction A ganglioglioma is a type of primary central nervous system low grade tumour composed of mixed populations of glial and neuroepithelial elements. They account for 0.4–2% of all intracranial tumours and appear more commonly in children and young adults. Seizures, which are the most important symptom in these tumours, improve significantly after surgical excision. Methods Between 1995 and 2008, 20 patients with (12 adults and 8 children) with intracranial ganglioglioma were treated at our hospital. Clinical information obtained by chart review included sex, age at onset of symptoms, clinical history, results of neurological examination, tumour location, CT and MRI appearance, surgical results and follow-up. All patients underwent tumour resection and the extent of surgery was determined from the surgical reports and postoperative imaging studies. Results The median age of patients was 26.4 years (range, 1–75 years), and the female to male ratio was 1.5:1. Except in one case, all patients had seizures with a median duration before diagnosis of 7.4 years (range 1–29). Seventeen tumours were located in the temporal lobe (9 right and 8 left). Macroscopically complete excision was performed in 17 patients and subtotal in the remaining 3. There were 4 cases of recurrence treated by surgery and radiotherapy being added in one case. The mean follow-up was 8.5 years (range 22 months–14 years) and disease free survival at 5 years was 85% and an overall survival of 95%. Conclusions The seizures, which are the most frequent symptoms, significantly improved after surgical removal. Surgery is the first choice of therapy in these tumours, and in the presence of subtotal resection or tumour recurrence the best indication for treatment is repeat surgery. Radiotherapy should be reserved only for malignant forms.

Infiltrative cerebellar ganglioglioma: conventional and advanced MRI, proton MR spectroscopic, and FDG PET findings in an 18-month-old child

Advanced magnetic resonance imaging (MRI) techniques, such as diffusion tensor imaging (DTI), dynamic susceptibility-weighted contrast-enhanced (DSC) perfusion, and proton magnetic resonance spectroscopy (MRS) have proven to be useful in predicting tumour grade and outcome in glial brain tumours. 1 Law M. Yang S. Wang H. et al. Glioma grading: sensitivity, specificity, and predictive values of perfusion MR imaging and proton MR spectroscopic imaging compared with conventional MR imaging. AJNR Am J Neuroradiol. 2003; 24: 1989-1998 PubMed Google Scholar , 2 Provenzale J.M. Mukundan S. Barboriak D.P. Diffusion-weighted and perfusion MR imaging for brain tumor characterization and assessment of treatment response. Radiology. 2006; 239: 632-649 Crossref PubMed Scopus (307) Google Scholar , 3 Stadlbauer A. Gruber S. Nimsky C. et al. Preoperative grading of gliomas by using metabolite quantification with high-spatial-resolution proton MR spectroscopic imaging. Radiology. 2006; 238: 958-969 Crossref PubMed Scopus (159) Google Scholar , 4 Weber M.A. Zoubaa S. Schlieter M. et al. Diagnostic performance of spectroscopic and perfusion MRI for distinction of brain tumors. Neurology. 2006; 66: 1899-1906 Crossref PubMed Scopus (182) Google Scholar , 5 Young R.J. Knopp E.A. Brain MRI: tumor evaluation. J Magn Reson Imag. 2006; 24: 709-724 Crossref PubMed Scopus (59) Google Scholar However, discrepancies do occur when advanced techniques suggest a biologically more malignant behaviour in low-grade lesions such as gangliogliomas. 6 Kincaid P.K. El-Saden S.M. Park S.H. et al. Cerebral gangliogliomas: preoperative grading using FDG-PET and Tl-201-SPECT. AJNR Am J Neuroradiol. 1998; 19: 801-806 PubMed Google Scholar , 7 Koeller K.K. Henry J.M. Superficial gliomas: radiologic–pathologic correlation. RadioGraphics. 2001; 21: 1533-1556 Crossref PubMed Scopus (111) Google Scholar , 8 Kumabe T. Shimizu H. Sonoda Y. et al. Thallium-201 single-photon emission computed tomographic and proton magnetic resonance spectroscopic characteristics of intracranial ganglioglioma: three technical case reports. Neurosurgery. 1999; 45: 183-187 Crossref PubMed Scopus (27) Google Scholar , 9 Provenzale J.M. Arata M.A. Turkington T.G. et al. Gangliogliomas: characterization by registered positron emission tomography MR images. AJR Am J Roentgenol. 1999; 172: 1103-1107 Crossref PubMed Scopus (22) Google Scholar , 10 Wang Z.Y. Sutton L.N. Cnaan A. et al. Proton MR spectroscopy of pediatric cerebellar tumors. AJNR Am J Neuroradiol. 1995; 16: 1821-1833 PubMed Google Scholar Recently, we had the opportunity to diagnose and follow a child with infiltrative cerebellar ganglioglioma. The purpose of this paper is to contribute to the existing body of knowledge about this rare entity by reporting and discussing the results of conventional and advanced MRI, including DTI, DSC, MRS, susceptibility-weighted imaging (SWI) and 2-[18F]-fluoro-2-deoxy-d-glucose positron emission tomography (FDG PET) with respect to the histopathological features of the tumour, and reviewing currently available literature.

Gangliogliomas intracraneales. Revisión de una serie de 20 pacientes

Introduction A ganglioglioma is a type of primary central nervous system low grade tumour composed of mixed populations of glial and neuroepithelial elements. They accounts for 0.4 to 2% of all intracranial tumours and appear more commonly in children and young adults. Seizures, which are the most important symptom in these tumours, improve significantly after surgical excision. Methods Between 1995 and 2008, 20 patients with (12 adults and 8 children) with intracranial ganglioglioma were treated at our hospital. Clinical information obtained by chart review included sex, age at onset of symptoms, clinical history, results of neurological examination, tumour location, CT and MRI appearance, surgical results and follow-up. All patients underwent tumour resection and the extent of surgery was determined from the surgical reports and postoperative imaging studies. Results The median age of patients was 26.4 years (range, 1-75 years), and the female to male ratio was 1.5:1. Except in one case, all patients had seizures with a median duration before diagnosis of 7.4 years (range 1-29). Seventeen tumours were located in the temporal lobe (9 right and 8 left). Macroscopically complete excision was performed in 17 patients and subtotal in the remaining 3. There were 4 cases of recurrence treated by surgery and radiotherapy being added in one case. The mean follow up was 8.5 years (range 22 months-14 years) and disease free survival at 5 years was 85% and an overall survival of 95%. Conclusions The seizures, which are the most frequent symptoms, significantly improved after surgical removal. Surgery is the first choice of therapy in these tumours, and in the presence of subtotal resection or tumour recurrence the best indication for treatment is repeat surgery. Radiotherapy should be reserved only for malignant forms.

Gangliogliomes multiples intracrâniens: À propos d’un cas

Multiple intracranial ganglioglioma (GG) is an extremely rare condition, generally associated with van Recklinghausen neurofibromatosis. We report the case of a 26-year-old woman who presented with progressive tetraparesis, raised intracranial pressure and visual loss. The neuroradiologic workup demonstrated a multiple lesion involving the temporal lobe, the diencephalomesencephalon and the optical pathways. Computed tomography-guided stereotactic biopsy of a right temporal lesion led to the diagnosis of GG. In light of this case and the literature review, we discuss morphologic aspects, differential diagnosis and therapeutic options of GG. In patients with multiple cerebral tumors, search for multiple ganglioglioma is particularly important because of the therapeutic implications and the good prognosis of this tumor.

Intracranial ganglioglioma: clinicopathological and MRI findings in 16 patients

To record the clinical findings and magnetic resonance imaging (MRI) characteristics of intracranial gangliogliomas in 16 patients. Sixteen patients were imaged using unenhanced and contrast-enhanced MRI. Eight patients underwent unenhanced CT and of these, three underwent contrast-enhanced CT. Two radiologists read the images retrospectively. The images were studied with regard to location, size, margin, signal intensity, enhancement characteristics, cystic changes, and presence of calcifications. Clinical data, such as presenting signs and symptoms, physical findings, and medical histories, were collected. Histopathological and immunohistochemical studies were performed and analysed by two pathologists. In 12 cases the tumours were located in one of the cerebral hemispheres; in the other cases they were located in the brainstem, cerebellum, suprasellar area or the thalamus. The tumour dimension varied from 1-7 cm, with a mean of 3.6 cm+/-1.8 cm. The MRI features of ganglioglioma in the present cohort can be divided into three patterns: cystic (n=2), cystic-solid (n=6), and solid (n=8). Solid lesions had a predilection for the temporal lobe; cystic and cystic-solid tumours had a wide anatomical distribution. Cystic lesions were significantly smaller than both cystic-solid and solid lesions (F=4.28, P<0.05). Cystic changes in the cystic-solid tumours showed one of the following patterns: those with walls showing contrast enhancement, those containing an enhancing nodule, or cysts without an obvious wall. The solid portion of cystic-solid gangliogliomas and the entire tumour in solid tumours showed homogeneous enhancement of variable degrees on T1-weighted (T1W) spin-echo (SE) images. Five tumours had mild or moderate oedema. In one patient two separate gangliogliomas were found, each lesion exhibiting different MRI features: solid and cystic-solid. One case of cortical ganglioglioma was found, causing bone erosion due to pressure. One tumour with chronic haemorrhage was found in the study. MRI features of gangliogliomas are non-specific. A ganglioglioma should be suspected when a tumour shows the following features: (1) a solid lesion located in the temporal lobes with mild or no oedema and homogeneous enhancement on SE T1W images; or (2) a small cystic lesion or cystic-solid mixed mass with a wall enhancement or a markedly enhanced nodule. We report a patient with two separate gangliogliomas and a case with bone erosion.

Intracranial ganglioglioma: preoperative characteristics and oncologic outcome after surgery.

In order to investigate the clinical characteristics, neuroimaging findings, pathological features, treatment, and outcomes, and to provide valuable guidance for the diagnosis and management of intracranial gangliogliomas, 34 cases of intracranial gangliogliomas were retrospectively analyzed. This study included 23 males and 11 females. Age at operation ranged from 17 days to 50 years. All patients had preoperative magnetic resonance imaging (MRI). Computed tomography (CT), magnetic resonance spectroscopy (MRS), 18F-FDG-PET (positron emission tomography), and 99Tc-HMPAO-SPECT (single photon emission computed tomography) were also performed in some cases. All pathological specimens and all available neuroimages were re-evaluated. The follow-up period varied from 12 to 89 months (mean 36 months). Seizure was the most common presenting symptom (85%). Tumor calcification was detected by CT scan in six of 11 cases. Seventeen gangliogliomas (50%) showed cystic components and 18 tumors (53%) were enhanced on MRI. All temporal lobe gangliogliomas were located intracortically and most of them had poor demarcation on MRI. In comparison with the contralateral normal area, the gangliogliomas showed a reduced Cho/Cr and NAA/Cr ratio, and an increased Cho/NAA ratio. On 18F-FDG-PET, gangliogliomas were usually hypometabolic. HMPAO-SPECT indicated tumor hypoperfusion or isoperfusion. A gross total resection was achieved in 25 patients. Tumor progression was observed in three patients who underwent an incomplete tumor resection. Two of them underwent a malignant transformation to a glioblastoma. Twenty-seven patients could carry on their normal life activity with the Karnofsky Performance Scale (KPS) of more than 80. Even though ganglioglioma is a slowly growing benign tumor, which could be demonstrated by magnetic resonance spectroscopy (MRS), PET, and SPECT, there is a chance of malignant transformation, especially in cases of incomplete tumor resection. Gangliogliomas should be resected gross totally, if feasible, to achieve the best long-term outcomes.

Thallium-201 Single-Photon Emission Computed Tomographic and Proton Magnetic Resonance Spectroscopic Characteristics of Intracranial Ganglioglioma: Three Technical Case Reports

Thallium-201 Single-Photon Emission Computed Tomographic and Proton Magnetic Resonance Spectroscopic Characteristics of Intracranial Ganglioglioma: Three Technical Case Reports

Intracranial ganglioglioma: CT and MRI findings.

Thirteen cases of pathologically proved intracranial gangliogliomas were reviewed in order to define the characteristic computed tomography (CT) and magnetic resonance image (MRI)-features. All patients were evaluated with CT and four of them were studied by MRI. Six tumors were cystic dominant mixed masses with remarkable focal contrast enhancement (6/13, 46%); and seven were solid in nature with variable contrast enhancement (7/13, 54%). Nine of them contained calcification (9/13, 69%). The temporal lobe was affected in six patients (6/13, 46%); the posterior fossa in four patients; the frontal lobe in two; the remaining huge one in the frontal, temporal and basal ganglion. On MRI, the findings were similar to those of the CT. Gangliogliomas do not have a characteristic CT and MRI features. They may appear as pure cystlike tumors, cystic dominant mixed tumors with remarkable focal contrast enhancement, solid tumors with variable contrast enhancement or diffuse involving the brain parenchyma and/or subarachnoid spaces. However, radiologists should include ganglioglioma in the differential diagnosis when we find patient has a long-standing process as well as a calcified tumor in the temporal lobe with following characteristic; cystic dominant mixed tumor with remarkable focal contrast enhancement, or a solid mass without hemorrhage or significant surrounding edema.

Gangliogliomas in adults

BACKGROUND Gangliogliomas are rare tumors occurring in both children and adults that are characterized by the presence of neoplastic cells resembling both neurons and glia. METHODS The authors reviewed 18 adult patients with intracranial gangliogliomas treated at the study institutions between October 1987 and November 1995. RESULTS The median age at diagnosis was 33.7 years, with a range of 21 to 55 years. Median follow-up was 37.7 months, with a range of 4 months to 13 years. Clinical manifestations among the patients included seizures (13), headache (2), ataxia (1), and hemiparesis and paresthesias (1 patient each). Tumors were located in the temporal lobe (6 patients), temporal lobe and thalamus (1 patient), frontal lobe (5 patients), cerebellum (4 patients), and insula or thalamus (1 patient each). Thirteen patients underwent gross total resection, 4 underwent incomplete resection, and 1 underwent only a stereotactic biopsy. Treatment modalities included surgery only, surgery plus radiation, and surgery plus radiation and chemotherapy. Median survival was 90.3 months, with a range of 14 months to 13 years. Three patients were dead at follow-up with a mean survival of 32.3 months. These patients showed anaplastic features in their pathology at initial surgery or surgery for recurrence. CONCLUSIONS In adults, gangliogliomas have a relatively favorable prognosis; however, the presence of anaplastic features predicts a worse outcome. Cancer 1997; 79:127-31. © 1997 American Cancer Society.

Gangliogliomas in adults

Gangliogliomas are rare tumors occurring in both children and adults that are characterized by the presence of neoplastic cells resembling both neurons and glia. The authors reviewed 18 adults patients with intracranial gangliogliomas treated at the study institutions between October 1987 and November 1995. The median age at diagnosis was 33.7 years, with a range of 21 to 55 years. Median follow-up was 37.7 months, with a range of 4 months to 13 years. Clinical manifestations among the patients included seizures (13), headache (2), ataxia (1), and hemiparesis and paresthesias (1 patient each). Tumors were located in the temporal lobe (6 patients), temporal lobe and thalamus (1 patient), frontal lobe (5 patients), cerebellum (4 patients), and insula or thalamus (1 patient each). Thirteen patients underwent gross total resection, 4 underwent incomplete resection, and 1 underwent only a stereotactic biopsy. Treatment modalities included surgery only, surgery plus radiation, and surgery plus radiation and chemotherapy. Median survival was 90.3 months, with a range of 14 months to 13 years. Three patients were dead at follow-up with a mean survival of 32.3 months. These patients showed anaplastic features in their pathology at initial surgery or surgery for recurrence. In adults, gangliogliomas have a relatively favorable prognosis; however, the presence of anaplastic features predicts a worse outcome.

Cortical architectural abnormalities and MIB1 immunoreactivity in gangliogliomas: a study of 60 patients with intracranial tumors.

Gangliogliomas are generally low grade neoplasms composed of mixtures of neoplastic glial and neuronal elements whose origin and exact nature are still controversial. We studied a series of 60 intracranial gangliogliomas looking for coexistent cortical architectural abnormalities (cortical dysplasia, microdysgenesis) and to determine if tumor behavior correlates with MIB1 (marker of cellular proliferation) labeling. The patients included 34 males and 26 females who ranged in age from 6 months to 55 years (mean 20 years). Thirty-eight tumors (63%) were located in the temporal lobe and 6 (10%) in the frontal lobe. Fifty-four patients (90%) presented with seizures (most with intractable epilepsy) and the duration of seizures ranged from 1 to 38 years (mean 14 years). In all cases, the predominant glioma component resembled a low grade fibrillary astrocytoma. In 14 tumors (23%), an oligodendroglial component was present. In one case, the glial component resembled an anaplastic astrocytoma. The tumors were characterized variously by perivascular chronic inflammation (N = 45, 75%), vascular proliferation (N = 36, 60%), granular bodies (N = 54, 90%), binucleated neurons (N = 36, 60%), calcification (N = 28, 47%), and cystic degeneration (N = 26, 43%). Meningeal involvement by tumor was observed in five (8%) cases. In 38 patients, sufficient tissue was resected to evaluate for the presence of concomitant cortical architectural abnormalities. Cortical architectural abnormalities were identified near to but clearly separate from the tumor in 19 (50%) patients. Only four patients including the anaplastic tumor died with tumor progression. MIB1 indices (positive tumor cells/1,000 tumor cells counted) in 54 cases ranged from 0 to 10.2 (mean 1.1 +/- 1.0).(ABSTRACT TRUNCATED AT 250 WORDS)

CT and MR Findings of Intracranial Ganglioglioma

Purpose : The purpose of this study was to evaluate clinical, CT and MRI findings of intracranialganglioglioma. Materials and Methods : All patient were between 9 and 30 years old , and were men. Four patientswith pathologically proved gangliogliomas were included in this study. CT and MR images were assessedretrospectively for location, type, density, signal intensity and contrast enhancement pattern of tumors. Results: All patients suffered from epileptic seizure of long history. The tumors were located in temporal lobe(3 cases)and frontal lobe(1 case). The type of tumor were solid (2 cases) and cystic(2cases). On the CT scan, the cysticlesions were hypodense and the solid lesions were isodense to normal brain parenchyme. Calcification within lesionwas noted in 3 cases. On the MR imaging, cystic lesions reveal high signal intensity on T2-weighted image(T2WI)and low signal intensity on T1-weighted image(T1WI). One of the solid lesion showed iso-signal intensity on T1WIand high signal intensity on T2WI. Another revealed high signal intensity on both T1WI and T2WI. The signalintensity of the calcifications(3case) was variable. Two cases showed signal void, one case revealed high signalintesnity on T1WI and low signal intensity on T2WI. In three patients, the lesions enhanced after intravenousadministration of Gd-DTPA. Conclusion : Ganglioglioma should be considered in the differential diagnosis when thechildren or young male with epilepy have a cystic mass with calcification or ill- defined solid mass , especiallyin temporal lobe on CT or MRI.

A clinicopathologic and immunomorphologic study of 13 cases of ganglioglioma

The authors present the clinical, histopathologic, and immunomorphologic data of 13 intracranial gangliogliomas. Preoperative computed tomography scans showed a commonly cystic tumor of variable density. Six tumors were completely excised and seven were subtotally resected. After a mean follow-up of 4.5 +/- 2.6 years, 11 patients are asymptomatic or only slightly incapacitated. All tumors were examined with a panel of neuronal and neuroendocrine markers. Immunoreactivity (IR) to anti-neurofilament polypeptide (clone 2F11) was observed in neuronal processes in ten cases and in neuronal perikarya in five. With anti-synaptophysin (clone SY38), IR was present along the lining of ganglion cell perikarya and processes in 11 tumors whereas staining of the perinuclear cytoplasm was prominent in two. IR to anti-chromogranin A (clone LK2H10) was observed within the neuronal perikarya in eight cases. Only one ganglioglioma of the brain stem showed IR for tyrosine-hydroxylase (clone 2/40/15) and dopamine-beta-hydroxylase in some neoplastic ganglion cells. In this study, synaptophysin was the most reliable neuronal marker. For immunocytochemical identification of neoplastic neurons in ganglioglioma as well as other tumors with neuronal differentiation the authors propose a panel of well-characterized monoclonal antibodies against neurofilament polypeptides, synaptophysin, and chromogranin A to support the histomorphologic diagnoses.

Intracranial ganglioglioma: MR, CT, and clinical findings in 18 patients.

Eighteen cases of pathologically proved intracranial gangliogliomas were reviewed to determine their MR, CT, and clinical characteristics. Seventeen patients were evaluated with contrast-enhanced CT and 14 were studied by MR imaging. Eight tumors were predominantly cystic; half of these demonstrated some contrast enhancement, and five contained calcifications. These cystic gangliogliomas were located, in order of decreasing frequency, in the cerebellum, temporal, frontal, and parietal lobes. Ten tumors were solid; eight of these showed contrast enhancement, and only one contained calcifications. Small cysts were present in one solid mass. Solid gangliogliomas occurred preferentially in the temporal lobes. On MR, the findings were nonspecific and reflected the CT findings. In one patient who received gadolinium-DTPA the lesion did not enhance. Clinically, all patients presented with nonfocal long-standing symptoms and all but three were alive an average of 18 months after the initial diagnosis. Pathologists are recognizing ganglioglioma with increasing frequency, and although its radiographic characteristics vary, it should be included in the differential diagnosis when the above-described findings are encountered.

Evaluation, Surgical Approach and Outcome of Seizure Patients with Gangliogliomas

A retrospective study of 15 children with intracranial gangliogliomas and intractable seizures revealed that tumors associated with seizure were located in the temporal and frontal lobes. These patients underwent lobectomy under electrocorticography. Mesial temporal sclerosis was identified in the hippocampus of the excised temporal lobe in 7 of 13 patients with temporal lobectomies. Eleven of the 15 children were seizure-free over a mean follow-up period of 4 years. The surgical approach to gangliogliomas requires careful pre-operative evaluation, including neuroimaging and electrophysiological study to locate seizure activity. Removal of epileptogenic brain as well as the ganglioglioma is recommended to optimize seizure control.